a. Overall goals
Our late results, so far, have been excellent. All children, even those who began with the longest gaps or had failed previous operations, have had a successful true primary repair. Those who are far enough out from the repair (usually a year), to allow complete resolution of anastomotic strictures and to have overcome oral aversion, are eating normally. One child came to us with a tracheostomy in place and although he now has a very satisfactory esophagus, he will not learn to eat until the tracheostomy can be removed. We believe this will be accomplished this summer. Oral aversion seems to be the most lingering barrier to eating a normal diet. Although oral aversion may require a great deal of effort by the parents, we believe that in all cases it will be solved.
As the testimony to how well they eat, we published our long term follow-up studies and found the weight of our patients was within the normal range. In fact, their weights slightly exceeded their range of heights (reference 5). The size distributions were almost precisely normal indicating good nutrition and growth and development.
All children were eating whatever they wished and, at the time the study was completed, no child two years after repair, still had a gastrostomy tube (reference 5). With the referral here of increasingly complicated cases of EA in children who arrive at a year of age or older, not all g-tubes have been out by the age of two.
b. Esophageal Function
The normal esophagus empties in to the stomach by a combination of muscular contraction (peristalsis) and gravity. A rhythmic wave of peristalsis which is propagated within the muscle itself, begins in the throat and passes down to the stomach. It has been believed that the vagus nerve provides the stimulus for the contraction wave. But, both older experimental studies as well as our clinical observations indicate that the contraction stimulus passes within the muscle itself. Unfortunately, the contraction impulse does not pass across a repair site. Consequently, wherever a primary repair has been done or a segment of esophagus removed and the ends joined together, the contraction wave will not pass smoothly to the lower portion of the esophagus.
The esophageal muscle below the repair site does have an intrinsic firing mechanism, however, and contractions will occur. The contractions they are not smoothly rhythmic and progressive. As a result, any child who has had an EA repair, are considered to have disordered function of the lower esophagus. The practical consequences, however, do not appear to be great. The contractions will empty the esophagus satisfactorily into the stomach and the children are able to eat whatever they wish. A normal diet can be enjoyed.
On the other hand, interpositions of colon and stomach have no peristalsis and can empty only by gravity. Therefore, these children may have significant eating difficulties and may not be able to eat whatever they wish or enjoy a normal diet.
c. GE reflux
In our opinion, follow-up evaluations should be done for GE reflux. Reflux is common, particularly after very long gap repairs. In our patients, fundoplications have been done in about 30% of the children and more often in the long gap patients. We recommend a fundoplication for significant GE reflux, because of the uncertainties of treatment by medications alone (reference 6). Whether or not fundoplication will eliminate the need for long term antacid medication remains to be seen. We still recommend, therefore, esophagoscopy and biopsies every 1-3 years to make sure significant inflammation of the esophagus has not developed. Because our final goal, which has been realized in a large majority of our patients, is to be medication free, this follow-up is necessary. More time will be needed to accurately answer how often the potential problems of GE reflux occur, particularly in the children who have had very long gap repairs.
d. The very long term
For the very long term, we do not foresee any problems with the esophagus after a true primary repair. There are patients who had a relatively straight forward repair of EA/TEF forty years ago. The function of their esophagus appears to be very satisfactory. This should be true even for the longest gap repairs, because of the great growth potential inherent in the esophagus of all babies. It should not matter that originally the esophageal pouches were relatively short. The growth potential should more than compensate for this beginning.
In summary, we have shown that true primary repair is possible, apparently in all EA patients. This esophageal repair will reliably allow the children to eat normally and not be dependent on a g-tube. We believe the benefits of this approach will only increase with time. There seems to be no evidence for late problems arising with the esophagus, however, the potential for difficulties from untreated GE reflux remain. Clearly, however, after a few years, even the very long gap repairs cannot be distinguished from normal by what they eat.